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Fifteen-minute consultation:

structured approach to management

of a child with recurrent croup

Vineeta Joshi,1 Vikas Malik,2 Omar Mirza,2 B Nirmal Kumar2

1Department of Paediatrics,Royal Albert Edward Infirmary,Wrightington, Wigan & LeighNHS Foundation Trust, Wigan,Lancashire, UK2Department of OtolaryngologyOtolaryngology and Head andNeck Surgery, Royal AlbertEdward Infirmary, Wrightington,Wigan & Leigh NHS Foundation

Trust, Wigan, Lancashire, UK

Correspondence toDr Vineeta Joshi, Department ofPaediatrics, Royal Albert EdwardInfirmary, Wrightington, Wigan& Leigh NHS Foundation Trust,Wigan, Lancashire WN1 2NN,UK; [email protected]

Received 25 April 2013Revised 1 October 2013Accepted 23 October 2013Published Online First14 November 2013

To cite:Joshi V, Malik V,Mirza O, et al. Arch Dis ChildEduc Pract Ed2014;99:9093.

ABSTRACT

Recurrent croup is a distinct clinical entity from

viral croup. It is not a specific diagnosis and its

presence should alert the clinician to explore the

underlying cause. We discuss an evidence-based

structured approach to management of a child

with recurrent croup.

INTRODUCTION

Croup is a relatively common viral infec-tion of larynx, trachea and/or bronchi thatis characterised by an acute onset ofbarking cough, hoarseness and stridor withor without dyspnoea, typically during Falland winter season. It is uncommon to havemore than one episode of croup in a yearin an immune competent child.1 Croupyepisodes which occur more frequently(more than two episodes per year) havebeen referred to as recurrent croup.2 3

Recurrent croup is not a specific diagnosisand its presence should alert the clinicianto explore what is the real diagnosis4

(table 1). Recurrent croup can be the pres-entation of an underlying intrinsic and/orextrinsic airway narrowing process (box 1).

A thorough history and physical exam-ination is an essential preliminary step inestablishing the primary or contributorycause of recurrent croup.

WHAT SHOULD YOU ASK IN

HISTORY?

1. Previous episodes: Age of onset, presence/

absence of viral upper respiratory tract

infection symptoms, duration of each

episode, interval between episodes, pres-

ence of wheeze and importantly the

response to treatment should be enquired.

Severity of previous episodes should be

assessed based on age of presentation, fre-

quency of episodes, need for urgent or

inpatient treatment and need for intub-

ation. Recurrent croup in a child age more

than 4 years has been suggested as a

marker of increased severity.5

2. Previous intubation:In the birth and neonatal

history, intubation may indicate acquired sub-

glottic stenosis or subglottic cysts, which pre-

dispose to recurrent croup symptoms.

3. Gastro-oesophageal reflux (GOR): Ask

about symptoms suggestive of GOR in

each case and if already on antireflux treat-ment, any subsequent improvement in fre-

quency or severity of croup episodes

should be enquired.

Studies report an evidence of gastro-

oesophageal reflux disease (GORD) in

47%100% of their patients with recurrent

croup.2 3 5 One study showed that children

with recurrent croup who had GOR pre-

sented at a younger age (mean age 6 months)

and had shorter interval between episodes

(mean interval period 3 months) as compared

with those who did not have reflux.5

GOR isnot a causative factor but may aggravate or

unmask pre-existing airway pathology.

4. Reactive airway disease (RAD) and atopy:

History of asthma, eczema and allergies

should be taken. Also, adequacy of treat-

ment with respect to symptom control

should be assessed.

Recurrent croup has been referred to as

spasmodic croup in a number of earlier

studies that postulate recurrent croup to be a

spectrum of airway hyper-reactivity like

asthma. These studies report RAD to be

present in 40.4%82% of patients with

recurrent croup.68 However, a cause and

effect relationship between RAD and

recurrent croup has not been established.

Kwonget al2 reported history of asthma in

76.5% (n=17) of their patients but failed to

note any improvement in croup symptoms

despite optimum management of asthma.

5. Foreign body aspiration: Laryngeal/

tracheal foreign body can masquerade as

recurrent croup leading to a delayed

diagnosis.911

BEST PRACTICE

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6. Hoarseness or weak cry: Persistent hoarse voice or weak

cry in between episodes may indicate vocal cord path-

ology such as vocal cord paralysis or recurrent respira-

tory papillomatosis.12

7. Chronic symptoms: Persistent cough, dyspnoea, dyspha-

gia and recurrent chest infections associated with recur-

rent stridor may suggest a pathology causing externalairway compression. Gormleyet al13 reported a delay of

6 weeks to 10 years in the diagnosis of congenital

cardiovascular abnormalities that present with such

chronic symptoms.

8. Family history: Family history of croup, allergy and

atopy should be taken. Pruikkonen et al14 reported a

history of croup among parents and siblings to be the

most important predictive factor for croup and its recur-

rence in a child.Apart from the above focused history, a full paediatrichistory covering all aspects of growth and develop-ment should be taken to enable a comprehensivemanagement.

FOCUSED EXAMINATION OF A CHILD WITH

RECURRENT CROUP

1. Assessment of airway and breathing: In a child with acute

symptoms, immediately assess for severity of croup. Stridor

at rest, use of accessory muscles, chest wall retractions,

tachycardia, requirement of oxygen and increasing tiredness

suggest significant airway obstruction. Note the quality ofstridor, voice, cry, cough and presence of wheeze.

Type of stridor can help to localise the site of pathology.

Inspiratory stridor points towards a supraglottic problem,

for example, laryngomalacia; biphasic stridor points

towards a glottic or subglottic problem, for example, sub-

glottic stenosis or vocal cord paralysis; whereas expiratory

stridor points towards a tracheal pathology, for example,

external compression of trachea or tracheomalacia.

2. General examination: Check if the child is pyrexial or

has a toxic appearance. No response to standard treat-

ment of croup in an unwell child with pyrexia suggests

bacterial tracheitis.

Assess for failure to thrive. Presence of failure to thrive

may suggest an underlying significant GORD.

Look for any distinctive craniofacial features suggesting an

underlying genetic abnormality that may be associated

with an anatomic or functional airway abnormality.

Laryngospasm due to hypocalcaemia resulting in recur-

rent stridor has been reported in children with rickets.15

Look for presence of cutaneous haemangiomas.

Haemangioma particularly in the head and neck area

(beard distribution) may be associated with subglottic

haemangioma contributing to recurrent croup symp-

toms.16 Up to 50% of patients with subglottic

Box 1 Predisposing factors for recurrent croupin children

Congenital Congenital subglottic stenosis Congenital cardiovascular abnormality Tracheo-oesophageal fistula

Laryngotracheal cleft

Tracheobronchomalacia Vocal cord paralysis Congenital tracheal stenosis Congenital goitre

Traumatic Acquired subglottic stenosis Subglottic cysts Airway foreign body

Inflammatory Gastro-oesophageal reflux disease Asthma

Allergy

Infectious Viral Bacterial

Tumour Subglottic haemangioma Lymphangioma Thyroid neoplasm Recurrent respiratory papillomatosis Thymoma Lymphoma Mediastinal mass

Table 1 Croup versus recurrent croup

Croup Recurrent croup

Frequency Usually one episode per year Two or more episodes per year

Aetiology Parainfluenza type-1, influenza, RSV Possible underlying predisposing cause that may be unmasked by viralinfection (see box 1)

Age 6 Months to 3 years, peak incidence at 2 years Any age, can occur 3 years

Season During increased viral activityFall and winter season Throughout the year

Symptoms Usually a prodrome of URTI followed by hoarseness,

barking cough, stridor,respiratory distress

Hoarseness, barking cough, stridor, respiratory distress (usually no

prodrome but symptoms made worse if concurrent URTI)Course Short lasting, usually 12 days Relapsing, remitting course; episode can persist over weeks

Response totreatment

Symptoms completely resolve with standard treatment Some or no response to treatment

RSV, respiratory syncytial virus; URTI, upper respiratory tract infection.

Best practice

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haemangioma have a cutaneous haemangioma in the head

and neck area.17

3. Chest auscultation: Reduced air entry may be present

due to an airway foreign body or due to external tra-

cheal compression from a mediastinal mass.

4. Signs of allergy or atopy: Presence of nasal congestion,

mouth breathing, eczema and wheeze may suggest an

underlying atopy and airway reactivity predisposing to

recurrent croup.Apart from above focused examination, a detailedpaediatric general and systems examination should becarried out to complete the assessment.

WHAT MANAGEMENT SHOULD YOU PROVIDE?

1. Investigations: Appropriate investigation should be

carried out based on history and examination with the

aim of ruling out any underlying condition contributing

to the recurrence of croup symptoms.

A. Radiological investigations:

X-ray chest/neck: may be helpful to identify a

mediastinal mass or a radio-opaque foreign body.

A normal frontal and lateral chest radiograph hasbeen shown virtually to exclude the diagnosis of a

vascular ring as a cause of stridor.18

Cardiac/chest CT/MR scan: may be required if

there is suspicion of external tracheal compres-

sion by a vascular ring or a mediastinal mass.

B. Direct laryngo-tracheo-bronchoscopy (DLTB):

DLTB is the most important investigation to

exclude anatomic airway abnormality predisposing

to recurrent croup such as subglottic cysts and sub-

glottic stenosis. It can also demonstrate findings

suggestive of GORD and extrinsic tracheal com-

pression from causes such as double aortic arch,innominate artery and mediastinal mass.

Anatomic airway abnormality has been reported in

25%100% of children with recurrent croup who under-

went bronchoscopy.2 3 5 1 9 20 Therefore, a careful con-

sideration of risk factors should be taken to guide

referral to otolaryngologist (box 2).

C Investigations for GOR: Testing for GOR will

depend on your own unit protocol.

D Blood tests: These are generally not helpful unless

bacterial tracheitis, mediastinal mass or hypo-

calcaemia is a consideration based on history,

examination and other investigations.

2. Treatment: Episodes of recurrent croup may or may not

respond to the standard treatment of viral croup that is,

oral dexamethasone with or without nebulised adren-

aline. No response or a partial response to standard

treatment warrants further investigation. Specific treat-

ment will depend on the underlying cause.

Bacterial tracheitis needs urgent attention andtreatment with broad-spectrum intravenous antibio-tics, as there is a high likelihood of the need forintubation.

Referral to a paediatric otolaryngologist is import-ant for airway assessment. Subglottic stenosis is acommon finding on DLTB in children with recurrentcroup. Subglottic stenosis is diagnosed by conven-

tional DLTB with the child breathing spontaneouslyand the airway is sized using endotracheal tubes ofincreasing diameter. Mild stenosis is found more com-monly and is usually managed conservatively whilethe higher grades require surgical intervention. Themost severe cases may even require laryngotrachealreconstruction. DLTB should ideally be performedwhen the child is free of respiratory tract infection.

A therapeutic trial of antireflux therapy can behelpful if there are supporting clinical features.Hoaet al reported a significant decline in the numberand duration of croup episodes in 87% of their

patients after 6

9 months of antireflux treatment.3

Even children who did not demonstrate reflux relatedchanges on endoscopy showed symptomatic improve-ment. Importantly, 50% of patients on antirefluxtherapy who had a second endoscopic examinationshowed clinically significant decrease in grading ofsubglottic stenosis.

Associated RAD and atopy should be appropriatelymanaged with bronchodilators, steroids, antihistamineand other specific drugs according to the condition. Itis suggested that if patients with recurrent croup areconsidered to have hyper-reactive airway, it would bejustified to treat them with inhaled corticosteroids,similar to patients with bronchial asthma.6 7

CONCLUSIONS

Recurrent croupy episodes should prompt a search foran underlying cause that may be unmasked by a viralinfection. A thorough history and examination shouldbe undertaken to investigate the underlying cause.Referral to paediatric otolaryngologist for DLTBshould be made after consideration of risk factors toassist in diagnosis and management.

Box 2 Indications for referral to an ENT surgeon

History of previous intubation Age less than 1 year or more than 4 years Multiple hospital admissions with croup No improvement in symptoms despite appropriate

treatment for gastro-oesophageal reflux or reactive

airway disease and atopy Suspicion of foreign body inhalation Patients with congenital anomalies Presence of cutaneous haemangioma Patients with a persistent weak cry or hoarse voice Patients who did not respond to standard treatment

of croup

Best practice

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Contributors All the authors have helped in evidence-basedsearch and writing up the manuscript.

Competing interests None.

Provenance and peer review Commissioned; externally peerreviewed.

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recurrent croupapproach to management of a child withFifteen-minute consultation: structured

Vineeta Joshi, Vikas Malik, Omar Mirza and B Nirmal Kumar

doi: 10.1136/archdischild-2013-303846November 14, 2013

2014 99: 90-93 originally published onlineArch Dis Child Educ Pract Ed

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